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P2 Receptor Antagonists Rescue Defective Heme Content in an In Vitro SLC25A38-Associated Congenital Sideroblastic Anemia Cell Model.
Int J Mol Sci
December 2024
Department of Biosciences, Biotechnology and Environment, University of Bari Aldo Moro, 70125 Bari, Italy.
Mutations in the SLC25A38 gene are responsible for the second most common form of congenital sideroblastic anemia (CSA), a severe condition for which no effective treatment exists. We developed and characterized a K562 erythroleukemia cell line with markedly reduced expression of the SLC25A38 protein (A38-low cells). This model successfully recapitulated the main features of CSA, including reduced heme content and mitochondrial respiration, increase in mitochondrial iron, ROS levels and sensitivity to oxidative stress.
View Article and Find Full Text PDFAcute Erythroid Leukemia Post-Chemo-Radiotherapy and Autologous Stem Cell Transplantation Due to Multiple Myeloma: Tracing the Paths to Leukemic Transformation.
Int J Mol Sci
July 2024
Department of Pathology, Faculty of General Medicine, University of Debrecen, 4032 Debrecen, Hungary.
Article Synopsis
- * AEL is characterized by specific genetic changes, including multi-allelic mutations, and can develop after treatments like chemotherapy and stem cell transplants, as seen in two case studies.
- * Several potential mechanisms for AEL's development post-treatment are proposed, including the presence of abnormal stem cells, survival of mutated cells after chemotherapy, and new mutations arising from treatment-related complications.
Autopsy findings of acute erythroid leukemia.
Autops Case Rep
May 2023
Postgraduate Institute of Medical Education and Research, Department of Histopathology, Chandigarh, India.
Acute erythroid leukemia (AEL) is an exceedingly uncommon but distinct hematological malignancy that shows neoplastic proliferation of erythroid precursors with maturation arrest and no significant myeloblasts. We describe an autopsy case of this rare entity in a 62-year-old man with co-morbidities. He underwent a bone marrow (BM) examination for pancytopenia during the first outpatient department visit, which revealed an increased number of erythroid precursors with dysmegakaryopoiesis suggesting the possibility of Myelodysplastic syndromes (MDS).
View Article and Find Full Text PDFNeuregulin-1/PI3K signaling effects on oligodendrocyte proliferation, remyelination and behaviors deficit in a male mouse model of ischemic stroke.
Exp Neurol
April 2023
Department of Histology and Embryology, Institute of Neuroscience, Wenzhou Medical University, Wenzhou 325035, Zhejiang, PR China. Electronic address:
In this study, we investigated the effect of neuregulin-1 (NRG1) on demyelination and neurological function in an ischemic stroke model, and further explored its neuroprotective mechanisms. Adult male ICR mice underwent photothrombotic ischemia surgery and were injected with NRG1 beginning 30 min after ischemia. Cylinder and grid walking tests were performed to evaluate the forepaw function.
View Article and Find Full Text PDFThe accumulation of miR-125b-5p is indispensable for efficient erythroblast enucleation.
Cell Death Dis
October 2022
Stem Cell and Regenerative Medicine Lab, Beijing Institute of Radiation Medicine, Beijing, 100850, China.
Erythroblast enucleation is a precisely regulated but not clearly understood process. Polycythemia shows pathological erythroblast enucleation, and we discovered a low miR-125b-5p level in terminal erythroblasts of patients with polycythemia vera (PV) compared to those of healthy controls. Exogenous upregulation of miR-125b-5p levels restored the enucleation rate to normal levels.
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